PANCE Blueprint EENT (7%)

Papilledema (ReelDx)


56 y/o with intermittent loss of vision in the right eye

Patient will present as → a 57-year-old male with a history of hypertension who complains of an acute onset of intermittent headaches and blurred vision of the right eye.

Papilledema is swelling of the optic disk secondary to increased intracranial pressure → classically bilateral but (as in the video example) may be unilateral

  • Optic disk swelling resulting from causes that do not involve increased intracranial pressure (eg, malignant hypertension, thrombosis of the central retinal vein) is not considered papilledema.
  • There are no early symptoms, although vision may be disturbed for a few seconds. Papilledema requires an immediate search for the cause

Diagnosis is by ophthalmoscopy with further tests, usually brain imaging and sometimes subsequent lumbar puncture, to determine the cause

  • MRI or CT scan of the head looking for a cause. Focus on finding intracranial pathology = tumor or bleed, cerebral edema, CSF outflow obstruction or overproduction
  • Increased opening pressure with lumbar puncture confirms increased intracranial pressure

Causes include the following:

  • Brain tumor or abscess
  • Cerebral trauma or hemorrhage
  • Meningitis
  • Arachnoidal adhesions
  • Cavernous or dural sinus thrombosis
  • Encephalitis
  • Idiopathic intracranial hypertension (pseudotumor cerebri), a condition with elevated CSF pressure and no mass lesion

Urgent treatment of the underlying disorder is indicated to decrease intracranial pressure.

  • If intracranial pressure is not reduced, secondary optic nerve atrophy and vision loss eventually occur, along with other serious neurologic sequelae.

Increased Intracranial Pressure (ICP)

Intracranial pressure (ICP) is a measure of the hydrostatic pressure in the brain. Three elements contribute to ICP: brain tissue, blood, and cerebrospinal fluid (CSF). While these elements usually remain in balance, factors such as an increased body temperature or increased arterial or venous pressures can cause the ICP to fluctuate. The Monro-Kellie doctrine states, that if one component of ICP (brain tissue, blood, CSF) increases, one of the other components will decrease to maintain a constant pressure. When this balance is disrupted or when compensatory mechanisms fail, increased ICP can result. Signs and symptoms of increased ICP include a change in the level of consciousness, headache, irregular respirations, widening pulse pressure, bradycardia, projectile vomiting, abnormal pupils, and decerebrate or decorticate posturing.

Increased Intracranial Pressure (ICP) Assessment
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Increased Intracranial Pressure (ICP) Interventions
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Question 1
A 19-year-old woman presents to the emergency department complaining of headache. The headaches are generalized and increasing in intensity. They have not responded to over-the-counter (OTC) medications. She complains of approximately 1 week of blurred vision, intermittent diplopia, and vague dizziness. Her medical history includes obesity and acne. She takes Accutane and oral contraceptives. She is found to have bilateral papilledema, visual acuity of 20/30 on physical examination, and a normal MRI of the brain. The next most appropriate step would be  
CT scan of the head
See B for explanation
lumbar puncture
therapy with high-dose prednisone
See B for explanation
stat cerebral arteriogram
See B for explanation
reassurance and follow-up in the office in 6 months
See B for explanation
Question 1 Explanation: 
The presence of headache associated with papilledema raises the concern for a brain tumor. The MRI excluded a mass lesion, raising a strong suspicion of pseudotumor cerebri. This is also known as benign intracranial hypertension. It is not a benign condition, however, since it causes severe headache and may result in visual loss. It is particularly frequent in obese adolescent girls and young women. The etiology is unknown but may be associated with the use of oral contraceptives, vitamin A, and tetracycline. The presentation consists of headaches caused by an increase in intracranial pressure and blurring of vision. There may be diplopia, but the remainder of the neurologic examination is unremarkable. Papilledema is virtually always part of the presentation. The mental status is normal. The differential diagnosis includes venous sinus thrombosis, sarcoidosis, and tuberculosis or carcinomatous meningitis. The last two are excluded by lumbar puncture. An abnormal cerebrospinal fluid is not consistent with pseudotumor cerebri. The diagnosis is made by excluding mass lesions with CT scan or MRI and demonstrating markedly increased intracranial pressure by lumbar puncture. The treatment involves weight loss, diuretics, and steroids. Repeat lumbar punctures to remove cerebrospinal fluid and decrease intracranial pressure are effective. In cases that are unresponsive to these measures, lumbar-peritoneal shunting is effective, as is unilateral optic nerve sheath fenestration. Effective treatment can improve headaches and prevent vision loss.
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References: Merck Manual · UpToDate

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